Systemic Mastocytosis

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Disease pathophysiology: It is often referred as mast cell disease. It can be caused in both in children as well in adults. People affected by mastocytosis are susceptible to itching, hives, and anaphylactic shock, caused by the release of histamine from mast cells. The symptoms are skin lesion, nausea, diarrhea, food and drug tolerance, ocular discomfort, headache, anaphylasis, malabsorption, ear nose throat inflammation, facial flushing, itching, a rapid heartbeat, abdominal cramps, lightheadedness or even loss of consciousness, etc.

Treatment: Acute anaphylaxis is treated with Epinephrine. Anaphylactic symptoms controlled by using H1 and H2 receptor blockers.Acute anaphylaxis can be treated with 0.3 mL of a 1:1000 dilution of epinephrine. In children, the dose is 0.01 mL/kg (up to 0.3 mL) administered every 10-15 minutes as needed. Corticosteroids have been used to control malabsorption, ascites, and bone pain and to prevent anaphylaxis.

Oral prednisone (40-60 mg/d) for 10-20 days has been used in the treatment of malabsorption. Cromolyn is also helpful for decreasing bone pain and headaches and for improving skin symptoms. Patients with osteopenia that does not respond to therapy may receive a trial of interferon alfa-2b. Primary disease can be treated successfully by chemotherapy. Statistics: The extrapolated prevalence was found to be 4,698,486 in Italy and the population esteemed used is double of 293,655,405.